Exaggerated elastin turnover in young individuals with Marfan syndrome: new insights from the AIMS trial.

ABSTRACT:

Aims

The fragmentation and loss of elastic fibre in the tunica media of the aorta are pathological hallmarks of Marfan syndrome (MFS) but the dynamics of elastin degradation and its relationship to aortic size and physiological growth remain poorly understood.

Methods and results

In this post hoc analysis of the AIMS randomized controlled trial, the association of plasma desmosine (pDES)-a specific biomarker of mature elastin degradation-with age and aortic size was analysed in 113 patients with MFS and compared to 109 healthy controls. There was a strong association between age and pDES in both groups, with higher pDES levels in the lower age groups compared to adults. During childhood, pDES increased and peaked during early adolescence, and thereafter decreased to l

SUBMITTER: Iskandar Z 

PROVIDER: S-EPMC10567063 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature